发色素性肾细胞癌（ChRCC）是第二常见的RCC变异组织学类型。在基因、基因组、代谢、起源细胞及对靶向和免疫治疗的反应方面，ChRCC与透明细胞型肾癌（ccRCC）有明显区别。ChRCC的发病机制尚不清楚，但目前的数据表明有两个潜在机制：通过PTEN通路突变引起的mTORC1过度激活以及导致氧化应激的线粒体功能失调。目前尚无特定的ChRCC治疗方法，但观察到针对酪氨酸激酶和mTOR抑制剂的一些反应。对免疫疗法的反应一般有限。最近发现包括固有淋巴细胞/IL-15和半胱氨酸稳态/铁死亡在内的可靶向通路。版权所有© 2023 Elsevier Inc. 保留所有权利。

Chromophobe renal cell carcinoma (ChRCC) is the second most common variant histology RCC. ChRCC is distinct from clear cell RCC (ccRCC) in terms of genetics, genomics, metabolism, cell of origin, and response to targeted and immune therapies. The pathogenesis of ChRCC remains unclear, but current data suggest two potential mechanisms: mTORC1 hyperactivation through PTEN pathway mutations and mitochondrial dysfunction leading to oxidative stress. There are no specific approved treatments for ChRCC, although some responses to tyrosine kinase and mTOR inhibitors have been observed. Response to immunotherapy is generally limited. Targetable pathways involving innate lymphoid cells/IL-15 and cysteine homeostasis/ferroptosis have recently been identified.Copyright © 2023 Elsevier Inc. All rights reserved.